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Optic nerve axons of retinal

ganglion cells

1.2 million nerve fibers .

ON sheath：

continuous with the meninges

dura、arachnoid and pia mater

<

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optic nerve functions

Ancillary Tests

1.Visual Field

2.Neuro-imaging

3.OCT

4.VEP

1.Visual Acuity2.Color Vision3.Pupil4.Contrast sensitivity

Optic nerve diseasesEtiology：

1.inflammation：optic neuritis

2 . ischemic optic neuropathy

3-Compression

4-Granuloma & infiltration

5-Hereditary

6-Toxic

7-Irradiation

8- Trauma

.

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Optic Neuritis

1-Retrobulbar neuritis

optic disc is normal. in adultswith multiple sclerosis.

2-Papillitis

hyperemia and edema

common in children.

3-Neuroretinitis

Papillitis

with macular star

least common type

viral infections

Swollen disc with or without peripapillary flame-shaped hemorrhages.

Rapid unilateral loss of vision

RAPD

Loss of color vision

Pain in moving the eye

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optic neuritisFig

Centrocecal scotoma

Bilateral optic neuritis

Bilateral

Central

scotoma

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Bilateral hemianopsia

MRI

demyelinating lesions

multiple sclerosis

Neuromylitis Optica

VF showed non-central scotoma

altitudinal VF

an ischemic mechanism play a role in ON in NMO patients

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Optic Neuritis Follow Up

Diffuse and central loss in the affected eye at baseline

Follow Up :

nerve fiber bundle defects were the predominant localized abnormalities in both the affected and fellow eyes

physicians evaluate the characteristics of optic neuritis and other optic neuropathies in the future

Ischemic optic neuropathy ( ION)

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Non- ArteriticArteritic

sudden painless loss of vision

hyperemic disc

Sectorial, diffuse edema

splinter hemorrhages

Subsequent optic atrophy

transient visual loss, temporal

pain, jaw pain, fatigue, weight loss

Pale disc

optic disc edema

of a chalky white color

GCA - vasculitishypoperfusion of ONH

Anterior ischemic optic neuropathy ( AION )

Nonarteritic ischemic optic neuropathy

visual field

altitudinal field defect

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NAION

sudden, painless visual loss OS shows AION . OD crowded disc

Normal VF ODUpper altitudinal loss OS.

small optic disk cup to disk ratio < 0.1classic “disk at risk”,

OD shows initial visual acuity of 20/20painless loss of vision

disk edema peripapillary hemorrhages.

inferior altitudinal defect Normal VF

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BILATERAL NA-AION FOLLOW UP

Bilateral inferior altitudinal defect

1.VF at baseline2.every 2 weeks

till resolutionof disc edema

3.then monthly till the fields stabilize(2- 6 month ). The normal fellow eye has a 15%

risk of NAION within five years

NAAION

OD day 1

OS day 11

OU day 90

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1 st. attack

At presentation

inferior ONH edema

FA :

leakage of the inferior ONH.

VF :

Inferior altitudinal field defect

OD: after 2 weeks

shows superior ONH edema.

OS: ONH is normal, crowded.

VF of OD :

Upper arcuate defect and stable inferior altitudinal defect.

2 month pale ONH

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Non-arteritic AION

•diffuse or sectorial edema

•Few splinter hemorrhages

•Resolution of edema and hemorrhages

•Optic atrophy and variable visual loss

VF : 2 weeks postoperatively

Bilateral posterior ischemic optic neuropathy

(PION) after lumbar spine surgery

Optic disc : 2 monthspostoperativelybilateral pallor

VF : 2 months postoperatively

C/O visual loss in the postoperative period normal fundus and normal Neuroimagingdiagnosis of PION

close follow-up is mandatory.

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PAPILLEDEMAEtiology1. Pseudo-tumor cerebri.2. Space-occupying intracranial lesions.3. Microbial infection: meningitis, encephalitis.4. Hypertensive encephalopathy5. Intracranial vascular lesions: sub-arachnoid hemorrhage, sagittal sinus thrombosis, etc.6. Head-injury - long standing contusions, carotid-cavernous fistula7. Metabolic disorders: Addison's disease, , hypo-parathyroidism, etc.8. Blood dyscrasias : leukemia, polycythemia, etc.9. Choroidal plexus tumour by hypersecretion.10. Miscellaneous; serum sickness, status epilepticus, sarcoidosis

bilateral and asymmetric . Hyperemic ONH.

PAPILLEDEMA

Loss of ONH margins

Flame-shaped hemorrhages

Disturbed axoplasmic flow

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Papilledema

Enlarged

blind spot

is a visual

field defect in

papilledema

VF 30-2 mild bilateral enlarged blind spot

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Pseudotumor Cerebri , idiopathic intracranial

hypertension ( IIH )

poor absorption

of CSF by meninges

patients with signs and

symptoms of increased

intracranial pressure

(ICP) of unknown cause

IDIOPATHIC ICH

VF :

in idiopathic intracranial hypertension.

Enlarged blind spot

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Double arcuate scotoma.

Severe visual field constriction

Patients with IIH should befollowed with sequentialquantitative VF fordecision making andprevent vision loss. .

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VF follow up in Papilledema Under medications

IIH with shunt surgery

Decision for surgery

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Medulloblastoma

Children brain tumor

Bilateral papilledema due to increased ICT

disc edema peripapillary splinter hemorrhage superonasally

Fundus :at presentation

optic disc edemabilateral (papilledema)

brain stem medulloblastoma

VF : bilateral nerve fiberbundle loss . 10 .21 dB-MD

M-10.21 dB 6 .11 dB-MD

M-10.21 dB

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Brain stem medulloblastoma

3 months after resectionof the tumor resolution of papilledema.

VF : 3 months after surgical resection

shows improved VF MD -6.9 dB OD -4.4 dB OS

6 .9 dB-MDM-10.21 dB

4 .4 dB-MDM-10.21 dB

10 .21 dB-MDM-10.21 dB

6 .11 dB-MD

Painful Loss of vision RAPD

Color vision defect

visual acuity ,RADDare normal.

Sudden painless loss of vision

optic disc edemaperipapillary hge

Optic neuritis papilledema NAION

optic disc edema hyperemia,

splinter hge.

Bilateral disc edemaSplinter hge ,Obliterated cup

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Optic nerve head tumors

Optic Nerve Melanocytoma

benign tumor made up of melanocytes and melanin

slowly grow

extend onto the surface of the optic nerve and invade the nerve fiber layer (feathered edge).

Optic Nerve Melanocytoma

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juxtapapillary Melanocytoma.

VF shows enlarged blind spot, due to tumor extension past the disc margin.

juxtapapillary Melanocytoma

Melanocytoma

Melanocytoma cause nerve fiber bundle defects that result from

compression at the optic disc.

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Capillary Hemangioma of the Disc

elevated, reddishmass

obscuring the opticnerve head

Dilated, tortuousfeeder vesselsarising from theoptic disc.

OPTIC NERVE Angioma

von Hippel-Lindau syndrome

Multiple angiomas

globular reddish with a dilated feeding artery and a tortuous draining vein

benign vascular neoplasm

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Optic nerve Angioma

VF: generalized

constriction

VF : before therapy

baseline visit

VF : Follow up after

antivascular endothelial growth factor ( AVEGF )

Astrocytic Hamartoma

Visual field shows isolated

enlargement of the blind spot

Multilobulated mulberry lesion

Benign glial cell tumor

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Drugs :

ethambutol, amiodarone, immunosuppressive medications as methotrexate and cyclosporine

Eating and social habits

drinking, smoking

Toxic Optic Neuropathy

5 weeks after onset of visual symptoms.

Bilateral optic nerves temporal pallor

toxicityEthambutol

monthly VF till the medication is discontinued.If visual field defects are seen, stop

drug and follow-up fields every 1-3 months till VF have either improved or stabilized

VF

3 months after onset of

visual symptoms.

Bilateral central scotoma

inferior temporal quadrant

defects

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OD normal VF OS superior altitudinal defect.

MRI shows enhancement of the retrobulbar portion of the left optic nerve

Anti- rheumatoid therapy

infliximab , Methotrexate

5-day history of decreased vision OS

A Congenital Anomalies

development of optic disc

Optic nerve hypoplasia

Optic pit

Optic disc drusen

Coloboma of optic nerve

Morning-glory syndrome

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DM / DD > 3 ONH is suspectedDM/DD > 4 Optic Nerve Hypoplasia

DM : DD = 3

Optic nerve Hypoplasia

DM:DD ratio diagnose ON hypoplasia.

Optic nerve hypoplasia

Small ONH due to a low number of axons.

double ring sign

Septo-optic dysplasia

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OPTIC NERVE HEAD HYPOPLASIA

All components are small

small disc areasmall cupsmall neural rim

Superior Optic Nerve Hypoplasia Topless disc

Segmental ON hypoplasia

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Superior entranceof CRV

Pallor of superiordisc

Superior Peripapillary halo.

Superior nerve fiberlayer is absent

Superior Optic Nerve Hypoplasia

VF : inferior arcuate defectsconsistent with superiorsegmental optic nerve

Hypoplasia

Congenital pit of the optic nerve

grayish circular pit along the temporal aspect of the optic cup.

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OPTIC PIT

Schisis-like cavity extending from

the optic disc to the macula & CME

Associated with serous sensory

detachment of the macula.

Optic pit

Corresponding upper field defect

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Acquired pit of the optic nerve

Acquired pit of the optic nerve. Note circular depression in the superior half of the optic cup.

Disk photo of APON

visual field of OD

from an NTG patient

Visual field of acquired optic pit

dense inferior nasal step and paracentral scotoma which corresponds to location of superior APON in the optic nerve.

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optic disc drusen

bilaterally elevated optic discs

with irregular scalloped margins

absence of central cup

ONHDNumerous round, yellowish elevations in ONH , OD

"lumpy-bumpy" yellow elevations within ONH, OS

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Autoflourescence in ONHD

Natural emission of lightby biological tissues.

late staining of the drusenis due to the localizedpressure of the drusendisrupting the bloodtissue barrier in the optic

nerve.

This scotoma represent early damage to the optic nervefrom buried optic nerve

drusen

OD : normal

(OS) shallow superiorarcuatescotoma

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superficial ONHD and peripapillary atrophy

OS :inferior altitudinal and

superior nasal defects.OD : severely constricted field loss

optic nerves gliosis.

Small optic cup

no visible drusen.

bilateral buried ON

drusen, confirmed by

ophthalmic ultrasound

Right normal VF.

Left superior arcuate

scotoma

Left OCT RNFL shows a

abnormal sector inferiorly

corresponds to the

superior arcuate scotoma.

This scotoma and RNFL

defect may represent early

damage to the optic nerve

from buried optic nerve

drusen (OND).

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No TTT for complications of ONHD.

Follow up by serial VF, OCT nerve fiber layer , and fundus photography.

Lower IOP to prevent further nerve fiber layer and optic nerve damage

optic disc drusen

Coloboma of optic nerve head

Inferonasal coloboma

of ONH,retina & choroid.

incomplete closure of the embryonic fissure.

white, bowl-shaped excavation of

the inferior ONH.

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Optic disc & choroidal coloboma

OS : choroidal coloboma inferior to the optic disc .

OCT shows that the sclera is bowed posteriorly

The optic disc is excavated because of the accompanying disc coloboma

Optic disc coloboma and visual field deficit mimic

glaucoma

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Bilateral coloboma of optic nerve

Optic disc duplication or coloboma

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Humphrey visual fields

Double blind spot

Superior hemifield defects

Morning Glory syndrome

Funnel -shapedexcavation

Of the posteriorpole

surrounded by anelevatedannulus of

Chorioretinal pigment .

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Megalo-optic disk

congenital anomaly, OS : normal disk

visual acuity 20/20 in each eye

DD glaucoma

DD morning glory

Inferior notch Megalopapilla

1-normal visual field 2- normal daily IOP curve.3- The cupping is symmetric vertically and horizontally4- No focal notching of the rim

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MEGALOPAPILLA AND MORNING

GLORY SYNDROME

Tilted Optic Nerve disc

optic nerve exits the eye at an oblique angle.

superiortemporal disc is raised, simulating disc swelling,

inferiornasal disc is flat or depressed .

The blood vessels enter the globe at an oblique angle.

bilateral

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Tilted Optic Disc

oval-shaped disc with the long axis at an oblique angle.

situs inversus of BV

peripapillary atrophy

Inferonasal Chorioretinal thinning and pigment accumulation

posterior staphyloma

VF show :

bitemporal upper

quadrantanopia,

mimic chiasmal lesion

it crosses the

midline –

diagnosis of

tilted disc

syndrome.

Bilateral tilted disc syndrome

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Right normal optic disc

Tilted Disc

left optic disc larger Temporal pigmentary changes atrophic infero- temporal regions

OS : superior arcuate scotoma

OD: normal

Myelinated nerve fiber layerwhite, feathery

patches follow the

NFL bundles striated

appearance

Normally myelination

of the optic nerve

fibers stops when it

reaches the optic disc

(shortly after birth).

1% myelination

extending a variable

distance into the

retina.

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Myelinated optic nerve fibers

enlarged blind spot

corresponding to the

area of myelination.

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Hereditary optic neuropathy

Dominant optic atrophy (DOA)

Mitochondrial diseasesmen and women alike have a50%chance of

transmitting the mutation to each child.

Death of

RGCs

Bilateral temporal optic disk pallor

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Humphrey V F

bilateral cecocentral

scotoma

bilateral super temporal field defect

Leber´s hereditary optic neuropathy

Mitochondrial inherited

women transmit themutation to all children,men do not

degeneration of retinal(RGCs) , their axons

. Disc hyperemia

. dilated capillaries

.Tortuous BV

.Swelling of peripapillarynerve fiber

. Bilateral optic atrophy

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Leber's hereditary optic neuropathy (LHON)

Central or cecocentral scotoma are the classic VF defects .

acute lossof central vision

Subsequentbilateral opticatrophy

Leber´s hereditary optic neuropathy

Quantitative VF is helpful

to know the natural history of

LHON and in understanding the

pathology and pathophysiology

of this disease ,

timing of gene therapy

normal VA at

baseline in the fellow eye

VF of normal eye shows

subclinical findings

worse rapidly over weeks to

months to similar cecocentral

scotoma

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COMPRESSIVE OPTIC

NEUROPATHY

Thyroid Eye Disease (Graves'ophthalmopath)

Autoimmune disease

Bilateral proptosisdiffuse enlargement of recti m.

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VF:after VF: under Thyroidectomy Medications

Take home message1.Visual filed test is critical in the diagnosis and

follow up of optic neuropathy.

2.Visual field defects in optic neuropathies take

several patterns including central, diffuse,

arcuate, and altitudinal defect.

3. The pattern of visual filed defect is not specific

of any etiology and any type of field defect can

occur with any optic neuropathy.

4. altitudinal defects are more common in

ischemic optic neuropathies

5.central, or cecocentral defects frequently

accompany toxic/nutritional and hereditary optic

neuropathies.

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